Difficulty breathing, shortness of breath to the effort, dry cough… After 60 years, these symptoms could indicate idiopathic pulmonary fibrosis (IPF). From October 5 to 11, 2015, World IPF week takes place and on this occasion, the French association Pierre Enjalran Fibrose Pulmonaire Idiopathique (APEFPI) wishes to remind people of the dangers of this pathology. “It is an invisible, irreversible disease with a short-term fatal outcome. It is therefore important to inform and break the silence around IPF. Patients, caregivers and those around them are not alone in the face of this pathology“emphasizes Françoise Enjalran, president of APEFPI.
Irreversible and fatal lung disease
FPI is a lung abnormalities, characterized by inflammation and irreversible scarring of the lung tissue around the alveoli. This phenomenon interferes with breathing, and reduces the volume of air that can enter the lungs: oxygenation of the blood is then more difficult, which causes shortness of breath and sometimes a bluish coloration of the lips and fingertips. The first symptoms are accompanied by loss of appetite and weight, and chronic fatigue. You should therefore not delay in consulting a doctor because the sooner the disease is detected, the better the effectiveness of the treatments. Indeed, “there are treatments available to slow its progression, to preserve the gradual decrease in respiratory capacity, and to live longer. It is essential that patients in whom this disease is suspected be seen earlier in specialized centers, for a precise diagnosis, appropriate management, and to continue research.“underlines Professor Vincent Cottin, pulmonologist at the Louis Pradel hospital in Lyon.
However, the diagnosis is problematic for doctors in almost 50% of cases, leaving patients with a long medical wandering. Indeed, the average time to diagnosis is one to two years after the onset of symptoms according to APEFP, while 50% of patients die two to three years after diagnosis.
Risk factors still poorly understood
IPF develops without an identifiable cause, as indicated by the adjective “idiopathic”. Certain factors such as smoking, prolonged inhalation of particles (wood or metal dust), gastroesophageal reflux or viral infections could play a role in triggering the disease. IPF primarily affects people between the ages of 60 and 70, and predominantly men. This disease affects up to three million people worldwide and around 9,000 in France, where 4,400 new cases are diagnosed each year.
From October to December 2015, APEFPI, in collaboration with the Reference Center for Rare Pulmonary Diseases and with the support of Boehringer Ingelheim laboratories, organizes information meetings in partner hospitals throughout France. For any information and to register for these events, go to the APEFPI Facebook page.
Source: press release from APEFPI, the Reference Center for Rare Pulmonary Diseases and Boehringer Ingelheim laboratories
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